AB0687 Prognostic meaning of right ventricular function and reserve in patients with systemic sclerosis
Authors: P. Xanthouli, J. Miazgowski, N. Benjamin, O. Gordjani, B. Egenlauf, S. Harutyunova, R. Seeger, A. Marra, N. Blank, H. M. Lorenz, E. Grünig, C. Eichstaedt
Journal: Annals of the Rheumatic Diseases (2022)

Background The delay of the diagnosis of pulmonary hypertension (PH) in patients with systemic sclerosis (SSc) leads to severe impairment of survival. Objectives The objective of this study was to investigate the prognostic impact of right ventricular (RV) function at rest and during exercise on survival in patients with SSc presenting for screening for PH. Methods Data from SSc patients who underwent routinely performed examinations for PH screening including echocardiography and right heart catheterization at rest and during exercise were analyzed. Uni- and multivariable analyses were performed to identify prognostic parameters in SSc and to compare them to known prognostic predictors. Results Out of 285 SSc patients screened for PH, 225 patients (81.3% female, mean age 58.1±13.0 years, 68% limited cutaneous SSc, WHO-FC II-III 74%, 24 manifest PH) were included in the analysis. During an observation period of 3.2±2.7 (median 2.6) years 35 patients died. Tricuspid annular plane systolic excursion ≤18 mm (p=0.0004), increase of cardiac index (CI) during exercise ≤ 2 l/min (p=0.0002), RV output reserve &gt;3 mmHg/l/min (p=0.001), peak CI ≤ 5.5l/min (p=0.01), pulmonary arterial compliance &gt;2 ml/mmHg (p=0.0005) and qualitative RV function at rest (p&lt;0.0001) significantly predicted survival. In the multivariable analysis, RV function at rest, diffusion capacity for carbon monoxide &lt; 65% predicted and CI increase &lt;2 l/min/m 2 were identified as independent prognostic predictors and had &gt;70% sensitivity and specificity to predict development of pulmonary vascular disease (PVD) during follow-up. Conclusion This study demonstrates that assessment of RV function at rest and during exercise may provide crucial information to identify SSc patients who are at a high risk of an impaired survival and development of PVD. References [1]Brown ZR, et al. Screening for pulmonary arterial hypertension in systemic sclerosis: Now or never!. Eur J Rheumatol. 2020;7(Suppl 3):S187-S192. Disclosure of Interests Panagiota Xanthouli Speakers bureau: MSD and OMT, outside the submitted work, Julia Miazgowski: None declared, Nicola Benjamin Speakers bureau: Actelion pharmaceuticals, Bayer HealthCare and MSD outside the submitted work., Ojan Gordjani: None declared, Benjamin Egenlauf Speakers bureau: Actelion, MSD, Bayer and OMT (less than $10,000 each)., Satenik Harutyunova Speakers bureau: Bayer, MSD, Actelion and GSK, outside the submitted work., Rebekka Seeger: None declared, Alberto Marra Speakers bureau: MSD, Norbert Blank Speakers bureau: MSD, GSK, Actelion and Bayer Vital., Hanns-Martin Lorenz Speakers bureau: AbbVie, BMS, Pfizer, Cellgene, Medac, GSK, Roche, Chugai, Novartis, UCB, Janssen-Cilag, AstraZeneca, and Lilly (less than $10,000 each) and research support from AbbVie, MSD, BMS, Cellgene, Medac, GSK, Roche, Chugai, Novartis, UCB, Janssen-Cilag, AstraZeneca, Lilly, Baxter, SOBI, Biogen, Actelion, Bayer Vital, Shire, Octapharm, Sanofi, Hexal, Mundipharm, and Thermo Fisher, Consultant of: AbbVie, BMS, Pfizer, Cellgene, Medac, GSK, Roche, Chugai, Novartis, UCB, Janssen-Cilag, AstraZeneca, and Lilly (less than $10,000 each) and research support from AbbVie, MSD, BMS, Cellgene, Medac, GSK, Roche, Chugai, Novartis, UCB, Janssen-Cilag, AstraZeneca, Lilly, Baxter, SOBI, Biogen, Actelion, Bayer Vital, Shire, Octapharm, Sanofi, Hexal, Mundipharm, and Thermo Fisher, Ekkehard Grünig Speakers bureau: Actelion, Bayer AG, and MSD; grants from GSK, Novartis, and United Therapeutics; and personal fees from SCOPE, OrPha Swiss GmbH, and Zurich Heart House, Consultant of: Actelion, Bayer AG, and MSD; grants from GSK, Novartis, and United Therapeutics; and personal fees from SCOPE, OrPha Swiss GmbH, and Zurich Heart House, Christina Eichstaedt: None declared